coenzyme a has been researched along with Amino Acid Metabolism Disorders, Inborn in 33 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 29 (87.88) | 18.7374 |
| 1990's | 2 (6.06) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 1 (3.03) | 24.3611 |
| 2020's | 1 (3.03) | 2.80 |
| Authors | Studies |
|---|---|
| Cances, C; Damaj, L; de Lonlay, P; Doummar, D; Espil, C; Fluss, J; François-Heude, MC; Kern, I; Lebigot, E; Leboucq, N; Lenaers, G; Meyer, P; Munnich, A; Roubertie, A; Roze, E; Spitz, MA; Torre, S; Touati, G; Warde, MTA | 1 |
| Bian, F; Brunengraber, H; Jin, Z; Kelleher, JK; Tomcik, K; Zhang, GF | 1 |
| Rhead, WJ; Tanaka, K | 1 |
| Brown, GK; Cotton, RG; Danks, DM; Fowler, K; Grimes, A; Hunt, SM; Mercer, JF; Rogers, JG; Scholem, R; Truscott, RM | 1 |
| Butterworth, RF; Qureshi, IA; Ratnakumari, L | 1 |
| Gravel, RA; Hsia, Y; Lam, KF; Scully, KJ | 1 |
| Bachmann, C; Baumgartner, ER; Brechbühler, T; Wick, H | 1 |
| Brandt, NJ; Brandt, S; Christensen, E; Gregersen, N; Rasmussen, K | 1 |
| Brandt, NJ; Brandt, S; Christensen, E; Gregersen, N; Gron, I; Rasmussen, K | 1 |
| Maggio, DC; Nyhan, WL; Sweetman, L; Weyler, W | 1 |
| Hähnel, R; Wysocki, SJ | 1 |
| Armitage, IM; Hsia, YE; Lipsky, SR; Ramsdell, HS; Rosenberg, LE; Tanaka, K | 1 |
| Ando, T; Kilroy, A; Nyhan, WL; Sweetman, L; Wadlington, WB | 1 |
| Goodman, SI; Markey, SP; Miles, BS; Moe, PG; Teng, CC | 1 |
| Goodman, SI; Kohlhoff, JG | 1 |
| Bachmann, C; Barth, PG; Dianzani, I; Gibson, KM; Hoffman, GF; Schrynemackers-Pitance, P; Schutgens, RB; Sherwood, WG; Stumpf, DA; Weismann, U | 1 |
| Bau, DC; Gompertz, D; Hughes, EA; Peters, TJ; Storrs, CN | 1 |
| Bartlett, K; Charpentier, C; Draffan, GH; Gompertz, D; Goodey, PA; Saudubray, JM | 1 |
| Mamer, OA; Tjoa, SS | 1 |
| Gompertz, D | 1 |
| Bartlett, K; Gompertz, D; Goodey, PA | 1 |
| Hillman, RE; Keating, JP | 1 |
| Brandt, IK; Clement, DH; Hsia, YE; Provence, SA | 1 |
| Draffan, GH; Gompertz, D | 1 |
| Budd, MA; Efron, ML; Isselbacher, KJ; Tanaka, K | 1 |
| Isselbacher, KJ; Miller, EM; Tanaka, K | 1 |
| Hsia, YE; Lilljeqvist, AC; Rosenberg, LE | 2 |
| Hinds, KR; Mudd, SH; Uhlendorf, BW | 1 |
| Ando, T; Barnes, ND; Donnell, GN; Nyhan, WL; Rasmussen, K | 1 |
| Finch, CA; Hansen, S; Love, D; Perry, TL | 1 |
| Hsia, YE; Lilljeqvist, A; Rosenberg, LE | 1 |
1 review(s) available for coenzyme a and Amino Acid Metabolism Disorders, Inborn
| Article | Year |
|---|---|
Inborn errors of organic acid metabolism.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Biotin; Carboxylic Acids; Chromatography, Gas; Coenzyme A; Crotonates; Female; Gestational Age; Humans; Hydroxy Acids; Hydroxybutyrates; Infant; Infant, Newborn; Isomerases; Leucine; Male; Malonates; Metabolism, Inborn Errors; Pedigree; Pregnancy; Prenatal Diagnosis; Propionates; Pyrrolidonecarboxylic Acid; Valerates; Vitamin B 12 | 1974 |
32 other study(ies) available for coenzyme a and Amino Acid Metabolism Disorders, Inborn
| Article | Year |
|---|---|
Movement disorders in valine métabolism diseases caused by HIBCH and ECHS1 deficiencies.
Topics: Abnormalities, Multiple; Amino Acid Metabolism, Inborn Errors; Chorea; Coenzyme A; Dystonia; Dystonic Disorders; Enoyl-CoA Hydratase; Humans; Leigh Disease; Movement Disorders; Thiolester Hydrolases; Valine | 2022 |
Compartmentation of Metabolism of the C12-, C9-, and C5-n-dicarboxylates in Rat Liver, Investigated by Mass Isotopomer Analysis: ANAPLEROSIS FROM DODECANEDIOATE.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Citric Acid Cycle; Coenzyme A; Dicarboxylic Acids; Fatty Acids; Glutarates; Humans; Liver; Malonyl Coenzyme A; Metabolism, Inborn Errors; Mitochondria; Myocardium; Oxidation-Reduction; Peroxisomes; Propionic Acidemia; Rats | 2015 |
Demonstration of a specific mitochondrial isovaleryl-CoA dehydrogenase deficiency in fibroblasts from patients with isovaleric acidemia.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids, Branched-Chain; Butyrates; Cells, Cultured; Coenzyme A; Fibroblasts; Humans; Mitochondria; Oxidoreductases | 1980 |
beta-hydroxyisobutyryl coenzyme A deacylase deficiency: a defect in valine metabolism associated with physical malformations.
Topics: Abnormalities, Multiple; Acyl Coenzyme A; Amino Acid Metabolism, Inborn Errors; Coenzyme A; Consanguinity; Cysteamine; Cysteine; Enoyl-CoA Hydratase; Humans; Infant; Male; Thiolester Hydrolases; Valine | 1982 |
Effect of L-carnitine on cerebral and hepatic energy metabolites in congenitally hyperammonemic sparse-fur mice and its role during benzoate therapy.
Topics: Adenosine Triphosphate; Amino Acid Metabolism, Inborn Errors; Ammonia; Animals; Benzoates; Benzoic Acid; Body Weight; Brain; Brain Chemistry; Carnitine; Coenzyme A; Dose-Response Relationship, Drug; Energy Metabolism; Genetic Linkage; Glutamates; Glutamic Acid; Glutamine; Hair Diseases; Ketoglutaric Acids; Lactates; Liver; Male; Mice; Mice, Mutant Strains; Ornithine Carbamoyltransferase; Pyruvates; Time Factors; Urea | 1993 |
Genetic complementation of propionyl-CoA carboxylase deficiency in cultured human fibroblasts.
Topics: Amino Acid Metabolism, Inborn Errors; Carboxy-Lyases; Cell Fusion; Cells, Cultured; Coenzyme A; Fibroblasts; Genetic Complementation Test; Humans; In Vitro Techniques; Mutation; Parainfluenza Virus 1, Human; Propionates | 1977 |
Acute neonatal nonketotic hyperglycinemia: normal propionate and methylmalonate metabolism.
Topics: Acute Disease; Amino Acid Metabolism, Inborn Errors; Child; Child, Preschool; Coenzyme A; Female; Fibroblasts; Glycine; Humans; Hydrogen-Ion Concentration; Infant; Infant, Newborn; Infant, Newborn, Diseases; Ketosis; Ligases; Liver; Male; Malonates; Methylmalonic Acid; Methylmalonyl-CoA Mutase; Mitochondria, Liver; Propionates; Racemases and Epimerases | 1975 |
Glutaric aciduria in progressive choreo-athetosis.
Topics: Acyl Coenzyme A; Amino Acid Metabolism, Inborn Errors; Athetosis; Cerebral Palsy; Child; Coenzyme A; Female; Glutarates; Humans; Metabolism, Inborn Errors; Oxidoreductases | 1978 |
Glutaric aciduria: clinical and laboratory findings in two brothers.
Topics: Amino Acid Metabolism, Inborn Errors; Cerebral Palsy; Child; Child, Preschool; Coenzyme A; Consanguinity; Glutarates; Humans; Hydroxylysine; Infant; Infant, Newborn; Lysine; Male; Oxidoreductases; Tryptophan | 1977 |
Deficiency of propionyl-Co A carboxylase and methylcrotonyl-Co A carboxylase in a patient with methylcrotonylglycinuria.
Topics: Amino Acid Metabolism, Inborn Errors; Biotin; Carbon Dioxide; Cells, Cultured; Coenzyme A; Crotonates; Fibroblasts; Humans; Kinetics; Ligases; Propionates; Skin | 1977 |
3-Hydroxy-3-methylglutaric aciduria: 3-hydroxy-3-methylglutaryl-coenzyme A lyase levels in leucocytes.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Child; Child, Preschool; Coenzyme A; Glutarates; Humans; Infant; Leukocytes; Male; Oxo-Acid-Lyases | 1976 |
[13C]Valine metabolism in methylmalonicacidemia using nuclear magnetic resonance: propinonate as an obligate intermediate.
Topics: Amino Acid Metabolism, Inborn Errors; Child; Coenzyme A; Humans; Magnetic Resonance Spectroscopy; Male; Malonates; Methylmalonic Acid; Propionates; Valine | 1975 |
Hyperglycinemia and propionyl coA carboxylase deficiency and episodic severe illness without consistent ketosis.
Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Amino Acids; Citrates; Coenzyme A; Creatinine; Crotonates; Glutamates; Glutamine; Glycine; Humans; Infant; Ketone Bodies; Ketosis; Ligases; Male; Propionates; Pyrrolidonecarboxylic Acid | 1975 |
Glutaric aciduria; a "new" disorder of amino acid metabolism.
Topics: Amino Acid Metabolism, Inborn Errors; Child, Preschool; Coenzyme A; Dietary Proteins; Female; Glutarates; Humans; Infant; Leukocytes; Male; Oxidoreductases | 1975 |
Glutaric aciduria: inherited deficiency of glutaryl-CoA dehydrogenase activity.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Child; Coenzyme A; Female; Fibroblasts; Glutarates; Humans; Leukocytes; Male; Oxidoreductases; Pedigree; Skin | 1975 |
Phenotypic heterogeneity in the syndromes of 3-methylglutaconic aciduria.
Topics: Amino Acid Metabolism, Inborn Errors; Coenzyme A; Glutarates; Humans; Hydroxymethylglutaryl CoA Reductases; Meglutol; Phenotype | 1991 |
Localisation of enzymic defect in propionicacidaemia.
Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Amino Acids; Citric Acid Cycle; Coenzyme A; Consanguinity; Erythrocytes; Esters; Fatty Acids; Glycine; Humans; Hyperglycemia; Infant, Newborn; Ketones; Ligases; Male; Malonates; Mitochondria; Pedigree; Propionates | 1970 |
A defect in l-isoleucine metabolism associated with alpha-methyl-beta-hydroxybutyric and alpha-methylacetoacetic aciduria: quantitative in vivo and in vitro studies.
Topics: Acetoacetates; Adolescent; Amino Acid Metabolism, Inborn Errors; Avidin; Butyrates; Carbon Radioisotopes; Child; Chromatography, Gas; Coenzyme A; Female; Glycine; Humans; Hydroxybutyrates; Isoleucine; Leukocytes; Male; Mass Spectrometry; Propionates; Time Factors; Tritium | 1974 |
2-Ethylhydracrylic acid: a newly described urinary organic acid.
Topics: Acyltransferases; Amino Acid Metabolism, Inborn Errors; Chromatography, Gas; Coenzyme A; Humans; Hydroxy Acids; Hydroxybutyrates; Isoleucine; Keto Acids; Malonates; Mass Spectrometry; Valerates | 1974 |
Evidence for the enzymic defect in beta-methylcrotonylglycinuria.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Biotin; Carbon Isotopes; Child, Preschool; Coenzyme A; Crotonates; Glycine; Humans; Infant, Newborn; Leucine; Leukocytes; Ligases | 1973 |
Beta-ketothiolase deficiency as a cause of the "ketotic hyperglycinemia syndrome".
Topics: Acetoacetates; Acidosis; Acyltransferases; Amino Acid Metabolism, Inborn Errors; Butanones; Carbon Radioisotopes; Child; Chromatography, Thin Layer; Coenzyme A; Dinitrophenols; Fibroblasts; Glycine; Humans; Hydrazones; Hydroxybutyrates; Isoleucine; Malonates; Propionates | 1974 |
Propionicacidemia (ketotic hyperglycinemia): dietary treatment resulting in normal growth and development.
Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Body Height; Body Weight; Carboxy-Lyases; Child; Coenzyme A; Diet Therapy; Dietary Carbohydrates; Dietary Proteins; Female; Follow-Up Studies; Glycine; Humans; Intelligence; Ketone Bodies; Propionates | 1974 |
The identification of tiglylglycine in the urine of a child with -methylcrotonylglycinuria.
Topics: Amino Acid Metabolism, Inborn Errors; Butyrates; Child; Chromatography, Gas; Chromatography, Thin Layer; Coenzyme A; Diet Therapy; Dietary Proteins; Glycine; Humans; Hydro-Lyases; Male; Mass Spectrometry; Valerates | 1972 |
Isovaleric acidemia: a new genetic defect of leucine metabolism.
Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Butyrates; Caproates; Child, Preschool; Chromatography, Gas; Coenzyme A; Fatty Acids; Female; Humans; Leucine; Male; Propionates; Valerates | 1966 |
Hypoglycin A: a specific inhibitor of isovaleryl CoA dehydrogenase.
Topics: Acidosis; Alanine; Amino Acid Metabolism, Inborn Errors; Animals; Butyrates; Caproates; Carbon Dioxide; Carbon Isotopes; Carnitine; Chromatography, Gas; Coenzyme A; Cyclopropanes; In Vitro Techniques; Isoleucine; Leucine; Male; Oxidoreductases; Plant Poisoning; Rats; Valerates; Vomiting | 1971 |
Vitamin B12-dependent methylmalonicaciduria: amino acid toxicity, long chain ketonuria, and protective effect of vitamin B12.
Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Child, Preschool; Coenzyme A; Diet Therapy; Dietary Proteins; Humans; Isoleucine; Ketones; Leucine; Leukocytes; Male; Malonates; Methionine; Nutritional Requirements; Propionates; Threonine; Valine; Vitamin B 12 | 1970 |
Deranged B 12 metabolism: studies of fibroblasts grown in tissue culture.
Topics: Amino Acid Metabolism, Inborn Errors; Carbon Dioxide; Carbon Isotopes; Coenzyme A; Fibroblasts; Folic Acid; Glutamates; Homocysteine; Humans; Hydroxocobalamin; Isomerases; L-Serine Dehydratase; Male; Malonates; Methionine; Methyltransferases; Propionates; Skin; Vitamin B 12 | 1970 |
Propionic acidemia in patients with ketotic hyperglycinemia.
Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Child; Child, Preschool; Chromatography, Gas; Coenzyme A; Female; Glycine; Humans; Infant; Ligases; Male; Propionates; Remission, Spontaneous | 1971 |
Methylmalonic aciduria. An inborn error leading to metabolic acidosis, long-chain ketonuria and intermittent hyperglycinemia.
Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Chromatography, Thin Layer; Coenzyme A; Dietary Proteins; Glycine; Humans; Infant; Intellectual Disability; Isomerases; Ketone Bodies; Ligases; Male; Malonates; Vitamin B 12 | 1968 |
Importance of the single case.
Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Coenzyme A; Glycine; Humans; Infant; Intellectual Disability; Ketone Bodies; Male; Malonates; Vitamin B 12 | 1968 |
N-acetylcystathionine: a new urinary amino-acid in congenital cystathioninuria.
Topics: Amino Acid Metabolism, Inborn Errors; Autoanalysis; Chemistry, Clinical; Child; Chromatography, Ion Exchange; Chromatography, Paper; Coenzyme A; Female; Humans; Hydro-Lyases; Pyridoxal Phosphate | 1968 |
Methylmalonic aciduria: metabolic block localization and vitamin B 12 dependency.
Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Carbon Dioxide; Carbon Isotopes; Coenzyme A; Humans; Hydro-Lyases; Infant; Isomerases; Leukocytes; Malonates; Metabolism, Inborn Errors; Molecular Biology; Mutation; Propionates; Pyridoxal Phosphate; Succinates; Vitamin B 12; Xanthurenates | 1968 |